Characterization of Visual Function, Interocular Variabilityand Progression Using Static Perimetry–Derived Metrics inRPGR-Associated Retinopathy

Investigative Ophthalmology & Visual Science

article

James J. L. Tee¹  Yesa Yang¹  Angelos Kalitzeos¹  Andrew Webster¹  James Bainbridge¹  Richard G. Weleber³  Michel Michaelides¹ 

[1] UCL Institute of Ophthalmology, University College London;Moorfields Eye Hospital;Casey Eye Institute, Oregon Health & Science University

关键词: retinitis pigmentosa;    genetic diseases;    static perimetry;    visual field;    RPGR;   

DOI  :  10.1167/iovs.17-23739

学科分类：社会科学、人文和艺术（综合）

来源: Association for Research in Vision and Ophthalmology

【 摘 要 】

PURPOSE. To characterize bilateral visual function, interocular variability and progression byusing static perimetry–derived volumetric and pointwise metrics in subjects with retinitispigmentosa associated with mutations in the retinitis pigmentosa GTPase regulator (RPGR)gene.METHODS. This was a prospective longitudinal observational study of 47 genetically confirmedsubjects. Visual function was assessed with ETDRS and Pelli-Robson charts; and Octopus 900static perimetry using a customized, radially oriented 185-point grid. Three-dimensional hillof-vision topographic models were produced and interrogated with the Visual Field Modelingand Analysis software to obtain three volumetric metrics: VTotal, V30, and V5. These wereanalyzed together with Octopus mean sensitivity values. Interocular differences were assessedwith the Bland-Altman method. Metric-specific exponential decline rates were calculated.RESULTS. Baseline symmetry was demonstrated by relative interocular difference values of 1%for VTotal and 8% with V30. Degree of symmetry varied between subjects and was quantifiedwith the subject percentage interocular difference (SPID). SPID was 16% for VTotal and 17% forV30. Interocular symmetry in progression was greatest when quantified by VTotal and V30, with73% and 64% of subjects possessing interocular rate differences smaller in magnitude thanrespective annual progression rates. Functional decline was evident with increasing age. Anoverall annual exponential decline of 6% was evident with both VTotal and V30.CONCLUSIONS. In general, good interocular symmetry exists; however, there was both variationbetween subjects and with the use of various metrics. Our findings will guide patientselection and design of RPGR treatment trials, and provide clinicians with specific prognosticinformation to offer patients affected by this condition.

【 授权许可】

CC BY|CC BY-NC-ND